Pseudo Lennox Syndrome

Classification according to ICD-10
G40.00	Pseudo-Lennox syndrome Benign atypical epilepsy
G40.01	CSWS (Continuous spikes and waves during slow-wave sleep) Bioelectric status epilepticus in sleep ESES [Electrical status epilepticus during slow-wave sleep]
ICD-10 online (WHO version 2019)

The pseudo-Lennox-Gastaut syndrome is a relatively rare form of epilepsy . It gets its name from the similarity of the seizures to those of Lennox-Gastaut syndrome .

Pseudo-Lennox syndrome usually begins between the ages of two and seven. Mostly children are affected who already have a slight general developmental disorder or partial performance disorders (which can worsen as the disease progresses); however, it can also occur in children with completely normal development and in the presence of brain damage.

root cause

If one disregards cases with existing brain damage, the occurrence is idiopathic (also called genuinely ) justified. This means that there is no known triggering cause. It is assumed that such epilepsies are based on an increased susceptibility to seizures of areas of the cerebral cortex during brain maturation in child development.

This differentiates the pseudo-Lennox syndrome from the Lennox-Gastaut syndrome, in which the cause is usually already known or can be identified through examinations. In contrast to the Pseudo-Lennox, there are also no purely tonic seizures (see below). The transitions between these syndromes are fluid, as is the case with Landau-Kleffner syndrome .

Types of seizures in pseudo-Lennox syndrome

Tonic seizures

The aforementioned tonic seizures are those in which the entire body of the person concerned becomes stiff during the seizure, which is caused by cramping of the skeletal muscles. This can take a few seconds to several minutes. The affected person loses consciousness, which often results in dangerous falls. Breathing stops briefly, sometimes causing the lips or face to turn blue. Often the view is turned upwards, the pupils dilate, the head can be bent and the mouth can be open. The arms are also often slightly bent and raised, and the hips can also bend. Sometimes the tongue bites due to the tension in the jaw. Nevertheless, you should never try to push anything between your teeth, as this can lead to far greater damage and even death by suffocation.

In pseudo-Lennox syndrome, such tonic seizures do not occur individually, but only in combination with other forms of seizure.

Variety of seizures

Otherwise, however, an unusual variety of seizure symptoms is characteristic of pseudo-Lennox syndrome. The most important of these are briefly explained below:

Rolando seizures

It often happens to Rolando seizures , which are quite often occurring seizure type in children. Occasionally the pseudo-Lennox syndrome develops from an initially existing classic Rolandic epilepsy.

Grand mal seizures (tonic-clonic)

In addition, generalized tonic-clonic seizures occur that affect the entire brain . These correspond to the classic grand mal seizure . This means that after a suddenly occurring tonic seizure phase, as described above, rhythmic twitches ( cloni from Greek "klonos" for "violent movement") of the flexor muscles occur in all limbs , the initially high speed of which increases in the course of the Seizure slowed down. In this clonic phase , breathing resumes, often accompanied by moaning noises. During such a generalized tonic-clonic seizure, wetting and / or defecation can occur. There may also be increased salivation. After the twitching subsides, the body relaxes. In most cases, the loss of consciousness lasts for a few minutes. This is usually followed by a deep, long sleep or, more rarely, a slow awakening. After the attack, a state of great tiredness and exhaustion usually persists for a long time, often combined with painful sore muscles. Headache may also appear. In some other forms of epilepsy, clonic seizures occur without the tonic phase described.

In tonic and tonic-clonic seizures, the sudden, unprotected fall can lead to serious injuries. Inexperienced witnesses of such an attack should clear the area around the person concerned from objects that the convulsions could push themselves on, but otherwise not try to intervene in the course of the attack.

If the entire attack lasts longer than a maximum of five minutes, an emergency doctor should be called, as in this case a status begins that can endanger the life of the person concerned. Brief grand mal seizures, on the other hand, should be observed as closely as possible during their course, as this can mean help for the attending physician, but they are not life-threatening. The relatives of those affected often have emergency medication on hand that can be administered in the event of status epilepticus and, in most cases, interrupts it.

Myoclonic seizures

Another type of seizure that occurs in pseudo-Lennox syndrome are myoclonic seizures . They are also characterized by muscle twitching. However, in contrast to clones, they appear only very briefly and very suddenly, shockingly. Usually they only last a fraction of a second, more rarely several seconds. They resemble the flinch, as occurs with sudden, severe fright. You do not have to cover the whole body, but can also limit yourself to individual muscles or muscle groups. Mostly they affect both halves of the body more or less symmetrically. Myoclonic jerks are usually very violent, which can lead to falls. Objects held in the hand can also be thrown away by the twitching. There can also be - non-rhythmic - series of such twitches ( myoclonia ). However, unlike clonic seizures, myoclonus persists in consciousness. They can also be differentiated by the fact that, in contrast to myoclonus, they run rhythmically. Activities that have been started can be continued after a myoclonus.

There are also non-epileptic myoclonic jerks , such as B. the physiological, so non-pathological twitching to sleep or the benign sleep myoclonus of the infant.

Atonic-astatic seizures

Atonic astatic seizures and atonic nodding attacks also occur in pseudo-Lennox syndrome. They are characterized by sudden generalized muscle relaxation ( atony ) that affects the whole body . An atonic-astatic seizure therefore shows itself as a sagging of the body or a buckling of the knees. Often the result is a fall, which is the namesake for the word part "astatic". Usually, however, the person affected gets up again immediately afterwards. In the case of an atonic nodding attack, however, only the head “nods” forward.

If the attack is preceded by a myoclonia, one speaks of a myoclonic-astatic attack. Even with these seizures, consciousness is mostly retained. However, they can also be accompanied by a short absence (see below).

Atypical absences

Also atypical absence seizures can be observed in pseudo-Lennox syndrome. Absences (from absence, French for "absence") are caused by a generalized epileptic excitation of the brain, which, however, is of a milder expression than is the case with the generalized tonic or tonic-clonic and clonic seizures described above. That is why this does not result in a complete loss of consciousness, but only in a clouding of consciousness, because individual structures of the brain remain unaffected in their activity despite the generalization. An old expression for this type of seizure is "small attack" or also Petit-Mal (French for "little evil").

Typical absences occur very suddenly. Affected people suddenly pause in their movement for a few seconds, no longer react to external stimuli such as speech or touch (which is how they can be distinguished from a physiological "absent mind", deeply absorbed in thought, daydreaming) and received a blank look and an expressionless face. Just as suddenly, full consciousness returns and those affected continue, often after a brief uncertainty, a pause at the interruption in their perception, in their activity interrupted by the absence. In most cases, they have no memory of the absence. If other seizure symptoms occur with the described simple absence (also called simple or bland absence), one speaks of a complex absence .

In the pseudo-Lennox syndrome, such classic absences occur in a special form, the atypical ("unusual") absence. With them, the transitions from and to the waking state are not so sudden, but are slower and creeping. The clouding of consciousness is also often less severe, so that a reduced reaction to speech can be maintained. They can also have side effects such as slight twitching of the face, for example in the form of a blink or twitching of the eyelid or the corner of the mouth. Sometimes they are also associated with myoclonus and atonia at the same time, as described above. Atypical absences show a different EEG pattern compared to typical absences, and also with the myoclonia that occurs at the same time, the EEG change that can be observed is different than with the classic myoclonic absences.

Status epilepticus

In pseudo-Lennox syndrome there is a tendency for states to occur . A status means that the seizures either last much longer than would normally be the case, or occur in continuous series so quickly in succession that the person concerned has no opportunity to recover in between. Basically, a status is possible for all types of seizures.

Grand mal status can result in death, for example, from prolonged respiratory arrest or heart failure. Other states are not life-threatening, but if they occur frequently or if they are not interrupted, they can lead to significant developmental setbacks. With an absence status, those affected can fall into a kind of twilight state for hours or even days, which is not always recognized as an epileptic status because they sometimes remain responsive to a limited extent and capable of erratic actions.

ESES or CSWS

In addition, an ESES , a bioelectrical status during sleep (“electrical status epilepticus during slow sleep”), also called CSWS or CSWSS (“continuous spikes and waves during slow sleep”), which is a constant epileptic condition , can occur as a further form of seizure Means discharge in the brain during sleep and can only be recognized by an EEG recording during sleep.

EEG changes

The EEG always shows a severe multifocal change in pseudo-Lennox syndrome, especially during sleep. A multifocal change means that the seizure occurs in several places, called herds, in the brain at the same time or one after the other. Sometimes the seizures are also secondary generalized (after starting in one focus, the seizure focus, they spread to the entire brain.)

Pseudo-Lennox syndrome is often associated with ESES (a bioelectrical status during sleep), which manifests itself in a characteristic change in the EEG during a recording during sleep. In particular, this uninterrupted, generalized hypersynchronous (i.e. epilepsy-typical) discharge during sleep as well as seizures can result in serious, permanent developmental disorders, including severe intellectual disability.

treatment

Adequate treatment of the pseudo-Lennox syndrome and especially of ESES is very important because of the risk of serious developmental deficits. But this is usually difficult. Therefore, a good half of those affected show considerable deficits in their intellectual development towards the end of puberty .

There are several anticonvulsants ( drugs used to treat epilepsy) that are used in pseudo-Lennox syndrome. They are initially used in monotherapy , but mostly in combination therapy with several drugs, as one alone often does not have a sufficient effect. Usually you start with the active ingredient sultiam . Thereafter, valproate or lamotrigine is usually chosen, sometimes also clobazam or suximide (e.g. ethosuximide ). Finally, corticosteroids (e.g. cortisol ) or ACTH can also be used.

Frequently accompanying the pseudo-Lennox syndrome and especially ESES massive, often aggressive behavioral abnormalities and hyperactivity occur which are not related to upbringing and for which educational measures are therefore only of limited effectiveness. These abnormalities can usually be alleviated somewhat by appropriate drug treatment.

forecast

Despite the associated dangers for the development of the affected child, the pseudo-Lennox syndrome is counted among the benign (benign) epilepsies. This is because, in most cases, people become seizure-free by the end of puberty . This in turn is an indication that a disturbed brain maturation is probably actually the cause, which improves in the course of the development spurts associated with puberty.

But even after the epilepsy has been free from seizures, most of the developmental problems that have arisen are irreversible, i.e. irreversible, so that, despite progress in development, after having been free from seizures, the affected person is usually severely impaired. This applies in particular to cognitive limitations that occur frequently, i.e. those in intellectual development. Nevertheless, there are - rarely - those affected whose intelligence remains at a normal level or even - very rarely - formally meets the criteria of giftedness .

literature

• Christoph Dittrich, University of Kiel: Clinical course of atypical benign partial epilepsy in childhood ("pseudo-Lennox syndrome") . 2000, OCLC 62033643

Web links

• Pseudo-Lennox-Syndrome and ESES at anfallskind.de by Helmut Volkers, Bremen
• Treatment guidelines d. Status epilepticus on AWMF -online

swell

1. ↑ anfallskind.de: Questions and answers about Rolando seizures
2. ↑ anfallskind.de: What are myoclonic seizures?

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !